Granulomatosis with polyangiitis (GPA) is manifested as ocular pathology in almost a third of patients, which can either be detected at the onset of the disease or occur in the late stages. The pathological process involves the orbit, sclera, episclera, cornea, conjunctiva, eyelids, nasolacrimal system, optic nerve, retina and choroid. Lesion of eye and its surrounding structures determines the severity of the pathological process, the patient’s quality of life and prognosis. The article presents a clinical case of a 38-year-old man with an unfavorable course of GPA, lesion to upper and lower respiratory tract, kidneys, and musculoskeletal system. The authors consider ocular manifestations in the context of a systemic disease. An ophthalmological examination of the patient revealed changes in sclera and choroid, which had not previously been described in detail in the literature. The patient underwent cataract phacoemulsification in the right eye with implantation of a posterior chamber intraocular lens. The dynamics of clinical manifestations of eye lesion in the near future is positive, but in the long term it remains doubtful due to pronounced changes in the orbit and adnexa of the eye against the background of ongoing small vessels vasculitis. Early consultation with an ophthalmologist and optical coherence tomography in patients with GPA are necessary at any stage of the disease. The clinical case is presented with consideration to the CARE 2021 recommendations.
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